caa a22 4500 477087515 CHVBK 20180405111507.0 cr unu---uuuuu 170330e19961101xx s 000 0 eng 10.1007/BF00179952 doi (NATIONALLICENCE)springer-10.1007/BF00179952 Inner ear defect similar to Alport's syndrome in the glomerulosclerosis mouse model Mpv17 [Elektronische Daten] [A. zum Gottesberge Meyer, A. Reuter, H. Weiher] The Mpv l7 mouse strain is a recessive transgenic mouse mutant that develops glomerulosclerosis and nephrotic syndrome at a young age. The phenotype results from a loss of function of a gene coding for a hydrophobic peroxisomal protein of 176 amino acids of 20 kDa following its destruction by retroviral integration. To investigate a potential effect of the missing Mpv 17 function on the inner ear light and electron microscopic investigations were performed on the inner ears of Mpv 17 mice and controls. These revealed degeneration of the stria vascularis and spiral ligament, loss of cochlear neurons and degeneration of the organ of Corti. The alterations observed here were similar to those described for Alport's syndrome, an inherited disorder characterized by progressive nephritis and neurosensory deafness. These findings indicate that although the molecular cause is different, the Mpv17 mouse model may share pathological mechanisms involved in patients with Alport's syndrome. At present the Mpv17 mouse appears to be a suitable animal model for this disease and may help to further elucidate the relationship between the kidney and the inner ear. Springer-Verlag, 1996 Stria vascularis nationallicence Spiral ganglion nationallicence Organ of Corti nationallicence Collagen nationallicence Transgenic mouse strain animal model nationallicence zum Gottesberge Meyer A. Research Laboratory, Department of ORL, Heinrich Heine University, Moorenstrasse 5, D-40255, Düsseldorf, Germany aut Reuter A. Research Center, Institute for Genetics, Karlsruhe, Germany aut Weiher H. Research Center, Institute for Genetics, Karlsruhe, Germany aut European Archives of Oto-Rhino-Laryngology Springer-Verlag 253/8(1996-11-01), 470-474 0937-4477 253:8<470 1996 253 405 https://doi.org/10.1007/BF00179952 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00179952 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- zum Gottesberge Meyer A. Research Laboratory, Department of ORL, Heinrich Heine University, Moorenstrasse 5, D-40255, Düsseldorf, Germany aut NATIONALLICENCE 700 1- Reuter A. Research Center, Institute for Genetics, Karlsruhe, Germany aut NATIONALLICENCE 700 1- Weiher H. Research Center, Institute for Genetics, Karlsruhe, Germany aut NATIONALLICENCE 773 0- European Archives of Oto-Rhino-Laryngology Springer-Verlag 253/8(1996-11-01), 470-474 0937-4477 253:8<470 1996 253 405 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer